Bilateral Mondini dysplasia with normal hearing.
Bilateral Mondini dysplasia with normal hearing.
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双侧 Mondini 发育不良，听力正常。
DOI：
10.1159/000276406
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发表时间：
1993
期刊：
影响因子：
--
通讯作者：
T. Uemura
中科院分区：
文献类型：
--
作者：
S. Komune;K. Nogami;H. Inoue;T. Uemura
报告1例37岁男性，蒙迪尼发育不良，听力正常。病人来我们诊所就诊时主诉身体不稳。纯音测听显示双耳听力水平正常。多导断层扫描和计算机断层扫描(CT)显示双耳前庭和外侧半规管增大，但耳蜗管和其他半规管形状正常。冷热试验显示左耳有严重的神经管麻痹(CP)，右耳有中度的CP，而主动头旋转试验显示在0.33和0.67赫兹的频率下，头-眼的协调性得以保持，但在1.0赫兹时，头眼的协调性略有恶化。患者的不稳定似乎是由于双侧半规管功能减退，这可能是由于柔道摔跤引起的隐匿性、反复的脑脊液耳漏。尽管听力正常的Mondini发育不良被认为是罕见的，但从系统发育学角度来看，这类患者的存在比想象的要多。
A 37-year-old man with both Mondini dysplasia and normal hearing is reported. The patient visited our clinic with a complaint of unsteadiness. Pure-tone audiometry showed a normal hearing level in both ears. Polytomography and computed tomography (CT) revealed enlargement of the vestibules and lateral semicircular canals in both ears but a normal shape of the cochlea and other semicircular canals. The caloric test indicated severe canal paresis (CP) in the left ear and moderate CP in the right ear, whereas the active head rotation test demonstrated that head-eye coordination was preserved at frequencies of 0.33 and 0.67 Hz but slightly deteriorated at 1.0 Hz. The patient's unsteadiness seemed to be attributable to a hypofunction of the bilateral semicircular canals, which may be due to insidious, repeated cerebrospinal fluid otorrhea caused by judo wrestling. Although Mondini dysplasia with normal hearing has been believed to be rare, phylogenetic consideration suggests that more such patients exist than has been assumed.